Efficacy of Peri- and Postoperative Treatment in 95 Patients with Von Willebrand’s Disease.

Von Willebrand’s disease (VWD) is the most common inherited bleeding disorder in humans, exhibiting quantitative and qualitative defects in the von Willebrand factor. To investigate the quality of treatment during the peri- and postoperative course of patients with VWD, we conducted a retrospective data analysis. We analyzed data of 95 patients with VWD who went for 173 surgical interventions in our center between 1981 and 2003. Peri- and postoperative courses with regard of bleeding complications were reviewd. 59 Patients had type1 VWD (two severe, three moderate, 54 mild). 16 patients had type 2 VWD (14 2A, one 2B, one 2N) and 11 patients type 3 VWD. Nine patients with type1 VWD had an additional factor deficiency (five FXII-, two FVII- and two FV-deficiencies). We analyzed the course of 54 orthopedic surgeries, 41 abdominal surgeries, 15 angiographies, 12 endoscopic surgeries, 10 ear-nose-throat interventions, 11 mamma surgeries, 10 neurological surgeries, 8 soft tissue surgeries, 7 thorax surgeries, 3 thyroid gland surgeries and 2 biopsies. Most patients (108/173) were treated with Humate P, 33 patients with DDAVP (33/173) and 9 patients received both products during the pre- peri- and postoperative period. Three patients received other plasmatic FVIII- concentrates, 20 patients had no therapy for their coagulation disorder. 16 patients (9, 2%) had peri- or postoperative bleeding complications. Most of them were seen in patients with VWD type 2B and 3 and in patients who underwent major surgery and who received only DDAVP. Humate P was used for 9 interventions (9/108 = 8, 3%) and DDAVP for four interventions (4/33 = 12, 1%) with bleeding complications, three patients had no therapy. Most of these complications were reported in thoracic surgery (2/7 = 28%), gynecological (3/11 = 27%) and otolaryngological surgery (2/10 = 20%). Retrospectively 15 bleeding complications could be explained by surgical technique, thromboprophylaxis, inefficient substitution therapy or additional thrombocytopenia. In conclusion our findings show a low frequency of bleeding complications in VWS patients with surgical interventions. Our evaluation reveals the necessity of special hemostaseologic care and laboratory controls for these patients. Substitution therapy should depend on type of VWD and type of surgical intervention. Especially during thoracic surgery, gynecological and otolaryngological surgery a careful peri- and postoperative hemostaseologic management as well as careful hemostyptic techniques of the surgeon are required.